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Somatostatinoma

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Neuroendocrine Tumours

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Abstract

Somatostatinomas are rare neuroendocrine tumours (NETs) that arise in the pancreas or duodenum. They are the fifth most common pancreatoduodenal NET (after insulinomas, non-functioning tumours, gastrinomas and VIPomas), with an incidence of 1 in 40 million [1–3]. They tend to be non-functioning, although they are rarely active and can produce a spectrum of symptoms called ‘somatostatinoma syndrome’ [4–6]. A degree of discord exists regarding the functional component of a somatostatinoma; some clinicians argue that a ‘syndrome’ of clinical symptoms must be present for the diagnosis of somatostatinoma and others that the diagnosis is purely immunohistochemical [7]. As most of these tumours are biochemically inert, they are either detected incidentally or by causing mass-effect pressure symptoms, and most will have metastasised by the time of diagnosis [6, 8, 9].

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Authors and Affiliations

  1. Department of Hepato-pancreato-biliary surgery, Bristol Royal Infirmary, Upper Maudlin Street, Bristol, UK

    James M. L. Williamson MBChB, MSc, MRCS

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  1. James M. L. Williamson MBChB, MSc, MRCS
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Correspondence to James M. L. Williamson MBChB, MSc, MRCS .

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Editors and Affiliations

  1. Department of Medical Oncology, Hacettepe University Cancer Institute, Ankara, Turkey

    Suayib Yalcin

  2. Department of Endocrine Oncology, Uppsala University Hospital, Uppsala, Sweden

    Kjell Öberg

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Williamson, J.M.L. (2015). Somatostatinoma. In: Yalcin, S., Öberg, K. (eds) Neuroendocrine Tumours. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-45215-8_16

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  • DOI: https://doi.org/10.1007/978-3-662-45215-8_16

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